Vulva + vulva cancer Flashcards
What is stage 1
1A
1B
I Tumor confined to the vulva
IA Lesions ≤2 cm in size, confined to the vulva or perineum and with stromal invasion ≤1.0 mm, no nodal metastasis
IB Lesions >2 cm in size or with stromal invasion >1.0 mm, confined to the vulva or perineum, with negative nodes
What is stage 2
Tumor of any size with extension to adjacent perineal structures (lower third of urethra, lower third of vagina, anus) with negative nodes
What is stage 3
3A1 + 3A2
3B1 + 3B2
3C
III Tumor of any size with or without extension to adjacent perineal structures (lower third of urethra, lower third of vagina, anus) with positive inguinofemoral nodes
IIIA 1. With 1 lymph node metastasis (≥5 mm), or
2. With 1–2 lymph node metastasis(es) (<5 mm)
IIIB 1. With 2 or more lymph node metastases (≥5 mm), or
2. With 3 or more lymph node metastases (<5 mm)
IIIC With positive nodes with extracapsular spread
What is stage 4 vulval cancer
4a 1 + 2
4b
Tumor invades other regional (upper 2/3 urethra, upper 2/3 vagina), or distant structures
Tumor invades any of the following:
1. upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to pelvic bone, or
2. fixed or ulcerated inguinofemoral lymph nodes
IVB Any distant metastasis including pelvic lymph nodes
Histology of vulval cancer
Concerning features
- Infiltrative growth pattern (compared to a pushing pattern) is associated with higher local recurrence
- Fibromyxoid stroma at the invasive edge is associated with poorer outcome
- Lymphovascular space involvement = increased recurrence
What Ix are needed for the work up of vulval cancer ?
- Cervical cytology, and colposcopy of the cervix and vagina, if applicable, due to the association of HPV‐related cancers with other squamous intraepithelial lesions.
- Full blood count, biochemical profile, liver profile, and HIV testing.
- Chest X‐ray.
- CT or MRI scan of the pelvis and groins may be helpful, especially for locally advanced tumors, to detect any enlarged lymph nodes in the groins or pelvis, erosion into underlying bone, or other metastases. In addition, CT or MRI could be useful in further treatment planning.
- 18F fluorodeoxyglucose (18F‐FDG) positron emission tomography with computed tomography (PET‐CT) can more effectively assess and detect inguinofemoral lymph node involvement compared with CT
What affects recurrence?
• aim for tumor free pathological margins of 8mm
• same site
o associated with margins of 8 mm or less
o mean 21 months later
• different vulva site
o Occurred later - 69 month interval
o likely lichen sclerosis related
o often second primary tumors
How to manage close margins ?
- Close margins (less then 5 mm) can be given radiotherapy - it is not possible to reexcise
- Can use brachytherapy - take care to avoid necrosis risk
Bartholin gland cancers
How common
What types of cancer
What immunochemistry
What is the pathophysiology
How is it managed
• Bartholin gland carcinoma
o rare, unclear what related to HPV
o Transitional, SCC from the duct and adenocarcinomas from the gland itself
o Diffuse and intense p16 expression consistent with HPV
o Tx radical hemovuvectomy + bilateral groin dissection - difficult to achieve margins and post op radiation may decrease recurrence
o Adenoid cystic lesions radical WLE is adequate and adjuctive RT if positive margins or perineural invasion
Malignant melanoma of the vulva
How to stage?
How common
How to Ix
- Second most common
- 10% vulval cancers
- All pigmented lesion should be biopsied, most involve the clitoris or labia minora, de novo or from existing naevus, Irregular, pigmented, recent change, c/o lump or bleeding
- Need to use the Clark or Breslow modification of the staging system not FIGO staging
What is the management of vulva malignant melanomas
Surgery is Tx of choice - radical wide local excision with margins of 1 cm - trend is more conservative as radical vulvectomy doesn’t improve survival
• no survival advantage to lymph node dissection but RCT for intermediate thickness cutaneous melanomas elective node dissection had better survival
• Sential node biopsy has a 15% false negative rate - not standard practice
Risk factors for treatment recurrence
- multifocal
- large lesions
- smokers
- immunocompromise
- positive surgical margins
- age
- raised solidarity lesions
- immunosuppression
- Previous tx to genital tract
Prognosis for vulvar cancer
Prognosis
• 5 year survival with no lymph node involvement is in excess of 80%
• Less then 50% if lymph nodes re involved
• 10-15% if iliac or other pelvic nodes re involved
• Multifactorial analysis of risk factors for SCC prognosis showed nodal status and primary lesion diameter only variables that matter
How to treat VIN
Treatment of VIN
• WLE - small lesion, lowest recurrence risk, 0.5-1cm margin, safe in pregnancy, histological dx
• Imiquimod cream 5% - 60% response rate - doesn’t cause scarring, topical, anaesthetic not required, use for large multifocal areas
• Local destruction - CO2 lazer, cryotherapy - less anatomical distorsion, can be used in pregnancy, single or multifocal confluent lesions, lacks assessment of occult invasion
• skinning vulvectomy
• Recurrence is 50% at 1 year
• No good evidence comparing txs
Define Incisional biopsy
A biopsy taken with the intent of securing a diagnosis only. This should ideally contain the interface between normal and abnormal epithelium and be large enough for the pathologist to be able to adequately provide evidence of substage (in stage I cases).
Define Excisional biopsy
A biopsy taken that includes all of the abnormal epithelium but does not provide a tumour-free zone of 1 cm (after fixation) on all dimensions. This would normally be performed in cases of vulval intraepithelial neoplasia (VIN) or when there is a low suspicion of invasive carcinoma and the operator wishes to limit the amount of cosmetic harm.
Define radical excision
An excision performed with the intent of achieving clearance of at least 1 cm (after fixation) on all aspect of the tumour(s). Depending on the site and size of the tumour, this could vary from a radical local excision to a radical vulvectomy.
DVIN - VIN differentiated type
Association
Who gets it
risk
•
o 5% premalignant conditons of the vulva
o Associated with LP / LS
o Older woman
o High risk of malignant transformation
o 60% SCC wth shorter time interval and higher recurrence rate
• HSIL of the vulva - VIN usual type
o HPV 16,18 o Smoking, immunosuppression o Warty basaloid types o Younger woman, 35-49 o Multifocal, less potential for invasive malignancy o Vaccination reduces incidence o Majority or VIN o Treatment is excision with 5mm border and 4 mm depth
LSIL of the vulva -
flat condyloma, HPV effect
VIN presentation
- Often delayed
- Generally 65 or older
- Lumps, burning, itch, irritation, pain, asymptomatic
- Lesions are red, white or pigmented, variable ,multifocal
- Enlarged groin nodes is a bad sign
- High index for suspicion and low index for biopsy
- Carefully examine for IN of the cx, vagina, perinanal area
How to prevent vulval cancer
- Primary: HPV vaccination also reduces the risk of vulval cancer
- no specific screening
- Secondary: Woman with squamous intraepithelial lesions on the cx vagina or anus should have inspection of the vulva
- Tertiary prevention: treatment of predisposing and premalignant lesions
Risk factors for VIN
Risks • Lichen sclerosis • High risk HPV (16) • Pagets disease • Melanoma insitu
Lymphatic drainage of the vulva
- Primarily inguinofemoral region
- Secondarily to the external and internal iliacs
- This drainage is shared from with the inferior third of the vaginal tube and the most external portion of the anus (below the anal sphincter)
- Drainage can be unilateral or bilateral
- If the lesion is close to the clitoris it can be drained into the iliac region
